Systemic lupus erythematosus (SLE) is a chronic, autoimmune, multisystem disease which may affect the eyes and/or visual system in up to a third of patients. Severe retinal vaso-occlusive diseases have been rarely reported as manifestation in patients with SLE. We report the case of a 35-year-old female treated for systemic lupus erythematosus for 6 months, presented a sudden loss of vision. Fundus examination and fluorescein angiography revealed severe retinal vascular occlusion. This has motivated the search for antiphospholipid antibody syndrome and this was confirmed without the presence of anticardiolipin antibodies. And the treatment consisted in a laser therapy. The purpose of this case report is to demonstrate that an ocular vascular event can reveal the disease and that its diagnosis is important because this disease generally affects young people and may endanger ocular and vital prognosis.